Objective To analyze the basic characteristics of 1885 inpatients with rare diseases in a 3A hospital from January 1 to December 31,2023,so as to provide reference for the management of diagnosis and treatment of rare diseases and the formulation of policies.Methods Based on the medical record homepage data of rare disease inpatients in a tertiary hospital in 2023,analyze the basic characteristics and hospitalization cost structure of rare disease inpatients.Results The hospital ad-mitted 1 885 inpatients with rare diseases in 2023,accounting for 0.81％of the total number of discharged patients,with a male-to-female ratio close to 1:1.Patients aged over 60 accounted for 45.57％,followed by patients aged 45-59(26.47％),patients aged 19-44(20.9％)ranked third,and patients under 18 accounted for less.Rare diseases cover 11 disease classification sys-tems and including 80 diseases,which accounts for 38.65％of the rare diseases in the catalogue.The top ten diseases accounted for 70.56％of the total number of patients,and the top three diseases were progressive fibrosing interstitial lung disease,idio-pathic pulmonary fibrosis and primary biliary cholangitis.Patients under 1 year old accounted for 0.08％,patients aged 2-18 years accounted for 5.19％,patients aged 19-44 years accounted for 19.55％,patients aged 45-59 years accounted for 26.62％,and patients over 60 years old accounted for 48.57％.There was a large difference in the male-female ratio in four diseases:idio-pathic pulmonary fibrosis,amyotrophic lateral sclerosis,Takayasu's arteritis,and primary biliary cholangitis.From the perspec-tive of cost structure,the top ten rare diseases account for a high proportion of diagnosis fees,medicine fees and consumables fees.Conclusion Patients account for a high proportion of rare disease inpatients in the hospital and have a wide coverage of disease types.The overall male-female ratio of patients with rare diseases is similar,but the characteristics of the diseases are dif-ferent.The characteristics of rare diseases determine the structural characteristics of hospitalization expenses.Hospitals should improve the discipline construction and provide more resources for the diagnosis and treatment of rare diseases.To make guide-lines for the diagnosis and treatment of rare diseases and strengthen the training of doctors,so as to realize the early diagnosis and treatment of rare diseases.The state should continue to promote access to medication for rare diseases.

Performance appraisal of public hospitals have given a guidance for the development of public hospitals at all levels.A Class A tertiary hospital reviewed the problems in the development of the hospital at the present stage and focused on the following four aspects:①insufficient fine management;②No clear orientation of discipline development;③The bottleneck of the improvement of medical operation efficiency;④New challenges in the reform of payment mode.The tertiary hospital launched a fine management practice in May 2022,in order to solve the problems by taking the Department of Surgery as a pilot area,laying the foundation for fine management through information system construction,improving the efficiency of medical operation through management process optimization,improving the overall competitiveness of disciplines through the construction of sub-specialty and Discipline Alliance and adjusting the performance appraisal index system to play the role of performance incentives.The measures effectively improve the overall capacity and efficiency of hospital medical services and help the hospital to achieve high-quality development.

Objective To analyze the basic characteristics of 1885 inpatients with rare diseases in a 3A hospital from January 1 to December 31,2023,so as to provide reference for the management of diagnosis and treatment of rare diseases and the formulation of policies.Methods Based on the medical record homepage data of rare disease inpatients in a tertiary hospital in 2023,analyze the basic characteristics and hospitalization cost structure of rare disease inpatients.Results The hospital ad-mitted 1 885 inpatients with rare diseases in 2023,accounting for 0.81％of the total number of discharged patients,with a male-to-female ratio close to 1:1.Patients aged over 60 accounted for 45.57％,followed by patients aged 45-59(26.47％),patients aged 19-44(20.9％)ranked third,and patients under 18 accounted for less.Rare diseases cover 11 disease classification sys-tems and including 80 diseases,which accounts for 38.65％of the rare diseases in the catalogue.The top ten diseases accounted for 70.56％of the total number of patients,and the top three diseases were progressive fibrosing interstitial lung disease,idio-pathic pulmonary fibrosis and primary biliary cholangitis.Patients under 1 year old accounted for 0.08％,patients aged 2-18 years accounted for 5.19％,patients aged 19-44 years accounted for 19.55％,patients aged 45-59 years accounted for 26.62％,and patients over 60 years old accounted for 48.57％.There was a large difference in the male-female ratio in four diseases:idio-pathic pulmonary fibrosis,amyotrophic lateral sclerosis,Takayasu's arteritis,and primary biliary cholangitis.From the perspec-tive of cost structure,the top ten rare diseases account for a high proportion of diagnosis fees,medicine fees and consumables fees.Conclusion Patients account for a high proportion of rare disease inpatients in the hospital and have a wide coverage of disease types.The overall male-female ratio of patients with rare diseases is similar,but the characteristics of the diseases are dif-ferent.The characteristics of rare diseases determine the structural characteristics of hospitalization expenses.Hospitals should improve the discipline construction and provide more resources for the diagnosis and treatment of rare diseases.To make guide-lines for the diagnosis and treatment of rare diseases and strengthen the training of doctors,so as to realize the early diagnosis and treatment of rare diseases.The state should continue to promote access to medication for rare diseases.

Objective To investigate the effect of 20‐HETE on the isolated myocardial ischemia reperfusion injury and to ex‐plore its underlying mechanisms .Methods Experiments were performed in isolated rat hearts subjected to 35 min of ischemia fol‐lowed by 40 min of reperfusion in Langendorff preparations .HET0016 (1 μmol/L) and various concentrations (10 ,30 or 50 nmol/L) of 20‐HETE were infused 10 min before the onset of ischemia and throughout the reperfusion period .Cardiac hemodynamic changes and myocardial contractility were continuously recorded with the Powerlab /8P system .Myocardial infarct size was meas‐ured by TTC staining .The level of ROS and the protein carbonyl content were determined by DHE fuorescence and DNPH method , respectively .Results Perfusion with HET0016 significantly improved myocardial ischemia reperfusion injury reduction in cardiac contractility ,after inhibited the production of 20‐HETE significantly reduced the occurrence of myocardial infarction area (P<0 .05) ,but exogenous join 20‐HETE aggravated I/R‐induced myocardial injury (P<0 .05) .Myocardial ischemia reperfusion injury significantly increased production of ROS and oxidative stress ,both of which were significantly inhibited by HET 0016 and enhanced by 20‐HETE administration(P< 0 .05) .Conclusion 20‐HETE stimulates ROS production and enhance protein carbonylation , which aggravates myocardial ischemia reperfusion injury .

Objective To explore the related risk factors of renal function after partial nephrectomy,and provide the reference for clin-ic. Methods The data of 31 patients with small renal cancer in our hospital from January 2010 to June 2014 were analyzed. Before and after the treatment,the relationship of renal glomerular filtration rate ( GFR) with the change and clinical pathological factors were analyzed,the single factor analysis used t test,multiple factors analysis adopted multi-factor unconditioned logistic regression analysis. Results Single fac-tor analysis results showed that renal function after partial nephrectomy was closely related to the age,diabetes mellitus,blocking time,the size of the tumor,renal volume reduction ratio,the difference was statistically significant (P<0. 05). Many factors of logistic regression showed that it was closely related to the age,blocking time,renal volume reduction ratio(P<0. 05). Conclusion The age,blocking time,renal vol-ume reduction ratio are risk factors for renal function after partial nephrectomy.

Objective To evaluate the clinical methods and results of endoscopic-assisted catheterization in the treatment of bulbous urethral injury . Methods The clinical data of 19 cases of bulbous urethral injury from July 2004 to September 2012 managed by ureteroscopic catheterization were retrospectively analyzed . Results The procedures were successfully completed in all the 19 cases.Foley catheters were removed in 4-8 weeks after the surgery and all cases had unobstructed ureter after the removal of the catheter.Urethral dilatation was done regularly for 4 times and all the cases were followed up for 6-12 months afterwards.The follow-up showed urinary flow rate was more than 15 ml/s, and no urethral stricture , urinary incontinence or other complications occurred.Erection of penis was not obviously affected compared with preoperative condition . Conclusion Endoscopic-assisted catheterization is effective in the treatment of bulbous urethral injury .